Prafulla Kumar Sharma

Penicillamine induced pseudoxanthoma elasticum with elastosis perforans serpiginosa.

Long term D-penicillamine therapy, especially when used to treat Wilsons disease has been shown to cause elastosis perforans serpiginosa, pseudoxanthoma elasticum perforans and other degenerative dermatoses. We report a 23-year-old male patient who presented with multiple firm papules, nodules over the neck, axillae, front of elbows for five years. He was a known case of Wilsons disease on long-term treatment with penicillamine for the past 12 years. The papulonodular lesions were non-tender and some were discrete while others were arranged in a circinate pattern. There was central scarring of the skin within the circinate lesions. In addition, there were several small yellowish papules on both sides of the neck which eventually became confluent to form plaques. Histopathology confirmed the diagnosis of elastosis perforans serpiginosa and pseudoxanthoma elasticum. He was treated with cryotherapy (using liquid nitrogen through cryojet) for former lesions. The lesions showed remarkable improvement after five sittings. Now the patient is under trientine hydrochloride (750 mg twice daily) for Wilsons disease.

Oral ulcer as an unusual feature of visceral leishmaniasis in an AIDS patient.

Leishmaniasis, a globally prevalent parasitic disease, occurs in three forms, viz, visceral, cutaneous and mucocutaneous. It is transmitted by female Phlebotomus sandflies. Human immunodeficiency virus (HIV) infection is increasing worldwide and several reports indicate a rising trend of VL / HIV co-infection, modifying the traditional anthroponotic pattern of VL transmission. India is one of the countries having the largest burden of leishmaniasis; nevertheless, there are very few HIV / leishmania co-infection cases reported so far. We report a 35-year-old homemaker infected with the human immunodeficiency virus; she presented with an oral ulcer. The investigations carried out on her revealed that she was afflicted by visceral leishmaniasis and the oral ulceration was a part of the same. This is only the second such case from the Indian subcontinent and more significantly from a non-endemic area.

Isonicotinic acid hydrazide induced anagen effluvium and associated lichenoid eruption.

A 32 year‐old woman developed generalised lichenoid eruptions on her body followed by diffuse loss of scalp hair of the anagen effluvium type. She was receiving several anti‐tubercular drugs, including rifampicin, isonicotinic acid hydrazide (INH), pyrazinamide, and ethambutol, for abdominal tuberculosis. INH, which is a leading cause of drug eruptions in the above group of drugs was withdrawn. However, the other antitubercular drugs were continued along with 40 mg of prednisolone in a single daily morning dose. The latter was discontinued slowly over a period of 10 weeks. There was complete recovery of hair loss and the regrowth started after 12 weeks of alopecia. Such anagen effluvium with lichenoid eruption following INH therapy has not been observed previously. The complete recovery from anagen effluvium is difficult to explain, but it could have been because of the early initiation of corticosteroid.

Cutaneous relapse of primary testicular non-Hodgkin lymphoma-The first sign of disease dissemination

1. Joshi R, Marwah HS. Extrusion of sebaceous gland into a blister of pemphigus vulgaris: an unusual processing artifact. Indian J Dermatol Venereol Leprol 2004;70:316-7. 2. Koch PJ, Mahoney MG, Ishikawa H, Pulkkinen L, Uitto J, Shultz L, et al. Targeted disruption of the pemphigus vulgaris antigen (Desmoglein 3) gene in mice causes loss of keratinocyte cell adhesion with a phenotype similar to pemphigus vulgaris. J Cell Biol 1997;137:1091-102. 3. Koch PJ, Mahoney MG, Cotsarelis G, Rothenberger K, Lavker RM, Stanley JR. Desmoglein 3 anchors telogen hair in the follicle. J Cell Sci 1998;111:2529-37. 4. Mahalingam M. Follicular acantholysis: a subtle clue to the early diagnosis of pemphigus vulgaris. Am J Dermatopathol 2005;27:237-9. 5. Veeranna S. Extrusion of sebaceous gland and hair follicle into a blister of bullous pemphigoid. Indian J Dermatol Venereol Leprol 2005;71:209-10.

Developmental retardation in postinflammatory elastolysis and cutis laxa.

Several treatments have been proposed for persistent lesions including oral doxycycline, corticosteroids, radiotherapy, cyclosporine, surgical removal, phototherapy and intralesional rituximab.[5] In our patient, corticosteroids produced a slight symptomatic relief and surgery was not suitable due to the extent of the lesions. Treatment with intralesional rituximab was contraindicated as the patient planned to get pregnant.

Wolf's isotopic nonresponse in healed herpes zoster in erythroderma.

217 Indian Journal of Dermatology, Venereology and Leprology | Volume 84 | Issue 2 | March-April 2018 References 1. Puizina‐Ivic N, Bezic J, Marasovic D, Gotovac V, Carija A, Bozic M. Angiosarcoma arising in sclerodermatous skin. Acta Dermatovenerol Alp Pannonica Adriat 2005;14:20‐5. 2. Fonder MA, Douglas DK. Angiosarcoma complicating systemic sclerosis: A case report. Cutis 2008;81:468‐72. 3. Kubota N, Fujisawa Y, Nakamura Y, Tanaka R, Saito A, Maruyama H. Angiosarcoma of the Scalp in a Patient with System Sclerosis. The Journal of Dermatology 2015:42:102‐4. 4. Carter B, Jaworsky C, Fox M. Systemic sclerosis associated angiosarcoma: A case report and review of the literature. Int J Dermatol Clin Res 2016;2(1):14‐7. 5. Arbiser JL, Larsson H, Claesson‐Welsh L, Bai X, LaMontagne K, Weiss SW, et al. Overexpression of VEGF 121 in immortalized endothelial cells causes conversion to slowly growing angiosarcoma and high level expression of the VEGF receptors VEGFR‐1 and VEGFR‐2 in vivo. Am J Pathol 2000;156:1469‐76. 6. Shah AA, Rosen A, Hummers L, Wigley F, Casciola‐Rosen L. Close temporal relationship between onset of cancer and scleroderma in patients with RNA polymerase I/III antibodies. Arthritis Rheum 2010;62:2787‐95. How to cite this article: Mendiratta V, Rana S, Manickavasagam S, Nangia A, Chander R. Cutaneous angiosarcoma in a patient with systemic sclerosis: First case from India. Indian J Dermatol Venereol Leprol 2018;84:214-7.

Multifocal cutaneous Rosai-Dorfman disease masquerading as lupus vulgaris in a child

Dear Editor, A 13-year-old girl presented to us with red plaques and nodules on her chest and ears for the past two years. The lesions gradually increased in size and were asymptomatic. The patient was otherwise healthy and had no significant past medical history. General physical examination was normal, with no lymph node enlargement. Cutaneous examination revealed multiple infiltrative erythematous plaques, nodules and scars on the right breast surrounding an area of central atrophy and hypopigmentation, which occurred due to the intralesional triamcinolone injections received by the patient from a local practitioner (Figure 1). There was a reddish-yellow nodule of 1cm in size on the pinna of the left ear (Figure 2). There was no mucosal involvement. Routine biochemical tests were normal. Histopathological examination revealed a dense pandermal infiltrate composed of histiocytes and admixed plasma cells, neutrophils, lymphocytes and multinucleate giant cells. Histiocytes had abundant eosinophilic to pale cytoplasm and occasionally an inflammatory cell was present within the cytoplasm suggestive of emperipolesis (Figure 3). Mild fibrosis was present intervening the infiltrate. Stain for acid fast bacilli (AFB) was negative. Immunohistochemical staining revealed that the histiocytes were positive for S-100, CD68 and CD163. CD1a staining was negative. Fungal and AFB cultures were negative. Based on the above findings a diagnosis of cutaneous Rosai-Dorfman disease (RDD) was made. Patient was advised excision of the lesions.

Distinct clinico-immunological profile of patients infected with human papilloma virus genotypes 6 and 11

Anogenital warts are primarily caused by Human Papillomavirus (HPV) type 6 and 11, which belong to the taxonomic family Papillomaviridae, genus alpha-papillomavirus and species 10. The presentation of the warts is varied and most of the patients have high recurrence rate of wart lesions. Studies had shown that an effective cellular immune response is required for the control of HPV infection. Here, we report distinct clinico-immunological profile of two patients presenting with venereal warts caused by HPV genotypes 6 and 11. The Case 1 manifested greater number of verrucous warts and case 2 had fewer subtle lesions. Further, evaluation of HPV antigen-specific cellular immune response revealed a robust T cell response against HPV6 peptide and a weak response against HPV11 in case 1. Interestingly, HPV genotyping revealed type 6 in case 1 with greater severity of infection and robust immune response against HPV6 peptide. In contrast, case 2 presented with milder infection and weak immune response and was positive for genotype 11. More extensive study with larger cohorts will strengthen our observation and could be relevant for designing immunotherapeutic adjunct strategies along with the standard treatment for rapid clearance of HPV infections in these patients. This communication reports immune status of two patients with venereal warts and their correlation with clinical presentation and the genotyping.

Impact of melasma on quality of life in Indian patients

Introduction: Melasma is a chronic acquired disorder of facial melanosis characterized by brown-black macules on sun exposed areas of face. Melasma has a severe impact on the quality of life (QOL) of the patients, causing emotional, psychological, and social stress. Aims and objectives: (1) To evaluate the impact of melasma on QOL using the Hindi adaptation of Melasma QOL (Hi-MELASQOL). (2) To study the relation between Melasma Area and Severity Index (MASI) score and Hi-MELASQOL. Materials and Methods: A total of 156 patients of melasma attending the dermatology outpatient department of our institute were included. Patients were subjected to assessment of MASI score and Hi-MELASQOL questionnaire consisting of 10 objective questions using a Likert-scale of 1 to 7. Results: Hi-MELASQOL did not correlate statistically with MASI score, age of onset of melasma or with its duration. No statistical significant difference was found among the Hi-MELASQOL scores of patients with different occupation, education levels, or marital status. In Hi-MELASQOL questionnaire, 36.54% cases were bothered due to the appearance of their skin due to melasma, 41.03% felt frustrated, 46.03% felt embarrassed, and 48.72% felt depressed. Conclusion: Impact of melasma as measured by Hi-MELASQOL revealed that patients with melasma felt frustrated, embarrassed, and depressed. Melasma has effect on interpersonal interactions in Indian patients. However, Hi-MELASQOL is independent of MASI score. Hence, QOL should be assessed in every patient of melasma and treatment plan should be devised taking into account the psychosocial and emotional stress.

Benign vulvar vestibular papillomatosis: An underreported condition in Indian dermatological literature

Indian Dermatology Online Journal | January-February 2017 | Vol 8 | Issue 1 63 Other differential diagnosis to be considered for such distinctive skin lesions are connective tissue nevi, localized amyloidosis, and papular mucinosis. Connective tissue nevus can occur at different sites as compared to the patterned distribution of skin lesions in Hunter’s syndrome. Lack of inflammation and pruritus would rule against amyloidosis. Mucinosis presents as translucent firm papules and nodules.[1] “Pebbled skin” is a pathognomic sign of Hunter syndrome and is not seen in other types of MPS. Recognition of this entity can provide a clue to diagnose milder forms of the disease as it can be the earliest clinical feature, which helps in counseling the family.